Mast Cell Disorders Forum

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http://mastcelldisorders.wallack.us/yabb/YaBB.pl General Mast Cell Disorders Discussion >> General Mast Cell Disorder Discussion >> systemic mastocytosis http://mastcelldisorders.wallack.us/yabb/YaBB.pl?num=1330135823 Message started by peter on 02/24/12 at 14:10:23

Title: systemic mastocytosis
Post by peter on 02/24/12 at 14:10:23

http://mastcelldisorders.lefora.com/
I came looking in 2009 to find out how i could help my selth
looking at the old site and going through the new one
I do not see much help for the people with a diagnosis of
systemic mastocytosis or clusters of clonal mast cells by bone marrow
i am now stable and back at work full time
with a sit-down aircon job
but if i was new here i think i would have the same problems now as
I had on the old site in 2009
IF ANY ONE WITH SYSTEMIC MASTOCYTOSIS BY WHO CRYTIREA
Has addvice that might help others could you post it
with your diagnosis i do not have mastocytosis of the skin or food problems other then GI] WE need addvice from people with the
SAME DIAGNOSIS
Peter

Title: Re: systemic mastocytosis
Post by DeborahW, Founder on 02/24/12 at 15:06:52

Peter,

I am very happy to hear that you are stable now! Yay! :)

I don't actually understand your question because many people posting on both forums are diagnosed with systemic masto. So, that qualifies as plenty of advice from people with systemic masto. Is there a sub category of masto types that you are specifically looking to hear from?

Title: Re: systemic mastocytosis
Post by Anaphylaxing on 02/24/12 at 15:24:59

Welcome Peter, so glad to hear you've improved!!

Yes there are heaps of people on here with a diagnosis of systemic masto.

There is a spectrum of us on here with other mast cell disease but we all share many similar clinical symptoms and take similar medications, so I find that I learn a lot from everyone!

Hope you can share your experiences with us and what helped you achieve stability! 8-)

Title: Re: systemic mastocytosis
Post by peter on 02/24/12 at 20:33:47

HI deb I have been told that the docters are having truble
working out the differnce betwen mcas and sm
that thay are geting confused
that I should only look at people with the same diagnosis
As there is a very big differnce. so if people with the same diagnosis
put what has worked for them and what has not worked it might help
hi Anaphylaxis yes there is a large spectrum of other mast cell disease
i had mcas for 3 years but now it is gone but the sm is not good
what worked for me was 2 years off work the normal pils
with zometa iv i think my mcas was reacuring anaphylaxis
from a wasp sting that took a cupple of years to setell
but there are different course for different people

Title: Re: systemic mastocytosis
Post by Joan on 02/25/12 at 07:48:42

Hi Peter,

I have SM, too, confirmed by WHO criteria and a bone marrow biopsy. I have the c-kit mutation and very elevated tryptase. My symptoms are usually well-controlled by daily meds and lifestyle. Much of the information on this site applies to all mast cell disorder patients, especially daily medicating, treatment of anaphylaxis, lifestyle changes that help symptoms, foods and medicines that are generally tolerated and which to avoid, how to approach surgery safely, etc.

In my opinion, one important difference is how we're followed by doctors and what types of regular testing should be done. SM patients (and some others) need to be checked at regular intervals to make sure nothing is becoming aggressive. Tryptase, CBCs, and other tests give doctors information that will tell them if the SM is stable (indolent), smoldering (SSM), or aggressive (ASM). With SM especially, patients need to be checked to rule out other blood disorders that can sometimes appear. Other blood tests can give them important information, such as whether patients have low vitamin D or B12, since we seem to have malabsorption troubles sometimes.

Testing for C-Kit or other mutations can provide information in case a patient needs chemotherapy treatment at some point. Some treatments are more effective in patients without mutation(s) and don't work on patients who have specific ones, and vice-versa.

Bone density tests show if there is osteoporosis from the SM. Bone X-rays show whether there are bone lesions that need to be watched. If there are a lot or they are large or if there are breaks, then more aggressive treatment might be necessary.

If the mast cells are not proliferating, then, in general, these problems don't occur. There are exceptions, of course, especially in patients who don't quite meet the diagnostic criteria for SM, but who do have some minor criteria. They may be heading towards SM, but just aren't far enough along to find it.

Other problems in SM patients that might need more treatment would be finding decreasing numbers of other blood cells because the mast cells are taking up too much space in the marrow. There also can be involvement in other organs, such as enlarging of the liver and spleen that can affect health. These things need to be watched.

I'm not a doctor, so know I've greatly simplified this subject and only scratched the surface. SM is a complex disease, and it requires experts to follow it and treat it properly. I believe SM patients can benefit from being seen by a hematologist who is familiar with it, as well as being followed regularly by allergists/immunologists who can keep medications optimized, order testing, and educate patients in feeling better. Patients with GI symptoms might benefit from seeing a GI specialist who knows about the effects of mast cells on the GI system.

If you feel you're not stable right now or that it's taking increasing medicines to keep yourself stable, you might want to talk this over with a mast cell expert. It might give you peace of mind to know, if you don't already, whether your SM is stable. If you felt better when you weren't working, is disability a possibility? I can't remember where you live, but if you have an SM diagnosis in the U.S., you probably will qualify.

There are several new medications in clinical trials right now (Midostaurin is one) that are hoped to help ASM patients with minimal side effects, if that should be relevant at some point. Hope this addresses some of your thoughts.

Title: Re: systemic mastocytosis
Post by peter on 02/25/12 at 19:09:57

Thank you joan important information easy to read
i have found that most of my symptoms are pain related and phisical stress on the body makes it worse

i am also looking at the upregulated immun system responce
posible caurseing the exta pain
not looking fowered to winter

Title: Re: systemic mastocytosis
Post by Anaphylaxing on 02/26/12 at 13:51:32

I totally agree with Joan! You are a wealth of info and have helped me a great deal.

Peter, you probably heard that they came out with an article recently trying to unify the diagnostic criteria for mast cell disease/activation to include MCAS, SM, CM etc. The treatments for all of these are similar (though individualized and sometimes directed by C-kit etc) unless aggressive, but it's the follow-up/monitoring that changes based on your diagnosis.

I also agree with Joan that seeing a mast cell doc is the way to go!

I hope there are better days ahead for you!

Ana :D

Title: Re: systemic mastocytosis
Post by peter on 02/26/12 at 18:46:29

HI anaphylaxing yes i have seen the articles and in march i think
things will change with the criteria sum metting happerning
we have a very good mast cell doc in sydney who looks arfter us very well as for me i have not been better for 5 years
i hope others with SM post what helps them so all new people can see
if thay were to UNIFY all mast cell disorders well that wont happen
as all diseasers have mast cell problems

Title: Re: systemic mastocytosis
Post by ruth on 02/26/12 at 20:29:56

Peter would you mind posting the name of the doctor in Sydney (I think I recall you saying previously that you are in Sydney or NSW?) or at least the hospital unit that you have found helpful and knowledgeable? There is not much information out there for Australians so it would be helpful.

Ruth

Title: Re: systemic mastocytosis
Post by peter on 02/27/12 at 20:03:01

hi Ruth joan is helping with that
i don:t know if he wants his name on web sites
i will PM any one who needs a good doc in OZ yust leve a note
peter

Title: Re: systemic mastocytosis
Post by ruth on 02/28/12 at 01:44:07

Thanks Peter I appreciate it. It's good to hear of a doctor in Aus who is handling this well. My elder son lives in Sydney and I may be looking for a consult for him in the near future.

Ruth

Title: Re: systemic mastocytosis
Post by Maiysa on 03/06/12 at 05:26:30

Thank you Joan for the great information. It answered some questions I have.

Title: Re: systemic mastocytosis
Post by peter on 03/08/12 at 12:53:27

http://www.danmedj.dk/portal/page/portal/danmedj.dk/dmj_forside/PAST_ISSUE/2012/DMJ_2012_03/A4397
Systemic mastocytosis revue
Degranulation of mast cells is not to be regarded as an ON-OFF mechanism

Title: Re: systemic mastocytosis
Post by peter on 04/03/12 at 20:25:44

I have been on Zometa for osteoprosis every 3 mounths for 2 years
now the osteo has got a lot better ?Do we cut back to every 6 or 12 mounths
i allso went off cromoglycate for 8 weeks to find out what would happen i had sum flushing for about a week but then it stoped
the pain got worse but not to bad
arfter 3 days the inflamed bowl came back BAD
arfter going back on it the IBD was gone arfter 3 days
the pains are back to nomal

Title: Re: systemic mastocytosis
Post by peter on 04/21/12 at 13:15:58

http://www.expert-reviews.com/doi/full/10.1586/ehm.11.81

Title: Re: systemic mastocytosis
Post by Joan on 04/21/12 at 14:49:14

Hi Peter,

Glad the meds are working for you! I don't know what the time limit is on Zometa, but you might be able to find out from the manufacturer's website or a bone metabolism specialist might be able to tell you. Some of those drugs can cause bone death in the jaw and other parts of the body if used to long, but I don't know if Zometa is one of them.

When you mentioned "on or off" degranulating, are you referring to mast cells "leaking?" That's what I believe. Even with meds, they can still leak mediators, just in smaller quantities than in "shockers."

If that's not what you meant, please explain.

Your doctor doesn't mind being named on the forum along with his office contact information. He does NOT want his email posted. If you prefer, you could PM people who ask for it. He also wrote that he does not consider himself a mast cell disorder expert, but we know he's been good in treating you.

Title: Re: systemic mastocytosis
Post by peter on 04/21/12 at 22:12:36

Hi joan zometa is one of the drugs that cause bone death
what the time limit is i dont know either i think i might go for 6 mounth
iv for a wile
now back on the cromoglycate and the IBD has gone agan
the on - off was a quote from the link i posted
and yes it is leaking mast cells all the time but the immune system
t-b-nk cells all-so how are you doing well i hope
peter

Title: Re: systemic mastocytosis
Post by peter on 05/04/12 at 15:19:53

http://ainotes.wikispaces.com/file/view/Contemporary+consensus+proposal+on+criteria+and+classification+of+eosinophilic+disorders+and+related+syndromes+-+JACI+2012.pdf

Title: Re: systemic mastocytosis
Post by Joan on 05/04/12 at 16:20:56

Today was stressful, but otherwise, I've been okay. I found that turmeric capsules, twice a day has really helped with inflammation. I'm going to try quercetin again, but had to take a break because of my stomach issues.

Dr. Theoharides will be in Denver tomorrow at the Colorado Support Group meeting, and he's speaking about the effectiveness of luteolin vs. gastrocrom for stabilizing mast cells. I'll post whatever he says that might be helpful for anyone.

Thanks for the info.

Title: Re: systemic mastocytosis
Post by cutty sark on 05/05/12 at 05:00:44

Joan wrote on 05/04/12 at 16:20:56:

Hello Joan

I would be interested to hear about that. I stopped gastrocrom as the benefits were minimal and too much faffing around with the capsules. I found a link for luteolin and see a german Company sells it in Europe. Anything licensed by Germany is considered 'good to go' elsewhere in Europe as their licensing laws are so tight. It's quite expensive so I might see if there is a version licensed in the UK and get it from my GP as we pay no prescription charges in Scotland.

Title: Re: systemic mastocytosis
Post by Anaphylaxing on 05/05/12 at 08:11:03

"faffing around" ha ha! Well put! There is a lot of that in my life these days ;)

Title: Re: systemic mastocytosis
Post by peter on 05/05/12 at 20:13:47

theses are the people we wont woaking for us

Vol. 159, No. 1, 2012

--------------------------------------------------------------------------------

Review

Evaluation of Mast Cell Activation Syndromes: Impact of Pathology and Immunohistology
H.-P. Hornya, K. Sotlara, P. Valentb

aInstitute of Pathology, Ludwig-Maximilians-University, Munich, Germany;
bDivision of Hematology and Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria

Address of Corresponding Author

Int Arch Allergy Immunol 2012;159:1-5 (DOI: 10.1159/000336374)

--------------------------------------------------------------------------------

--------------------------------------------------------------------------------

Abstract

Mast cell activation syndromes (MCAS) are clinically defined disease states with a largely unknown morphological background. Since mastocytosis may be associated with MCAS, it is crucial in every patient to document or exclude mastocytosis by appropriate histological, molecular, and serological investigations of tissues/organs that are commonly involved in mastocytosis like skin, mucosa of the gastrointestinal tract and bone marrow. Accordingly, histopathological investigation including immunohistological stains is crucial to reach the final diagnosis in such patients and to classify MCAS into primary MCAS, which can present with or without evidence of overt mastocytosis, or secondary MCAS, where an underlying disease with or without tissue inflammation is detected. Cases without evidence of mastocytosis, monoclonal mast cells, or any underlying disease should be termed idiopathic MCAS. When the activating point mutant KIT D816V is detectable but criteria for diagnosis of mastocytosis are not completely met, a so-called (mono)clonal MCAS as a subvariant of primary MCAS should be diagnosed.

Title: Re: systemic mastocytosis
Post by WendyH on 05/10/12 at 07:38:56

Peter I am new to this forum but definately not new to SM. I was told mine is now more aggressive. In 2005 I had to stop the Gleevec because it damaged my liver and then they found out I had the mutation in the CKIT 816. Would have been nice if MD Anderson checked for that first. But anyway. I have had several bone marrow biopsies and meet the WHO criteria so its not like I am looking for a diagnosis. Not everyone's symptoms are the same and not everyone is affected by the same things. My mast cells are positive for cd2 and cd25 which was explained to me by my oncologist means they are malignant. Anyway, I suffer from severe bone pain, muscle spasms in arms and legs, nausea, diarrhea, extreme stomach bloating, and the most severe migraines, also extreme fatigue, brain fog, confusion. I am one of the rare mastos that can't take ketitofen or cromylin, I have every adverse reaction to it. So I am on Allegra 180 2x a day (H1), Zantac 300mgs 2x a day (h2), cymbalta (helps with the bone and muscle pain), b12 injections weekly (pernicious anemia from the masto) LDN 4.5 mgs a day, benedryl and epi pens as needed since I have lots of anaphylaxis for no reason. I also flush all the time and it feels like my insides are on fire and occassionaly feels like my blood gong thru my veins is molton lava. I have no allergies at all. So when I hear people refer to masto as an allergic disease it makes me soo made they have no clue what it is.

Title: Re: systemic mastocytosis
Post by peter on 05/10/12 at 21:10:36

HI wendy I started this blog for people with SM hoping to find
people like you your post will help a lot Joan is a lot better communicator then I the fire in the gut i now well
i take all my pills with milk seems to help a bit
I copied your post to the new doc as a ?? hope you dont mind
peter

Title: Re: systemic mastocytosis
Post by peter on 05/11/12 at 11:36:47

TO Wendy-joan and all with clonal systemic mastocytosis
i have pm deb and arsked her to start a new blog for all with who critirea
systemic mastocytosis sum where to post with out confusion
with allergies plese repost your storyes and help deb set it up
this is not to be seen as confertaion gust to stop confusion
when i first came here i was so messtup and the strees made it worse
a LOT worse and it is still happening to others please fix it let the people with disease tell there storys without CONFUSION

Title: Re: systemic mastocytosis
Post by peter on 05/11/12 at 14:51:41

Current state of biology and diagnosis of clonal mast cell diseases in adult -----------------------------
shttp://onlinelibrary.wiley.com/doi/10.1111/j.1751-553X.2012.01427.x/full
BY HEMATOLOGY free
use this one deb